Leivaditis Vasileios, Dahm Manfred, Papatriantafyllou Athanasios, Keul Hans-Georg, Kohl Lydia, Schäfers Hans-Joachim
Department of Cardiothoracic and Vascular Surgery, Westpfalz-Klinikum, Kaiserslautern, DEU.
Department of Pathology, Westpfalz-Klinikum, Kaiserslautern, DEU.
Cureus. 2024 Sep 26;16(9):e70241. doi: 10.7759/cureus.70241. eCollection 2024 Sep.
Liposarcomas (LPSs) are rare malignant tumors of adipocytic origin, primarily occurring in the extremities and retroperitoneum, with thoracic involvement being exceptionally rare. This case report details the surgical management and outcomes of a recurrent intrathoracic LPS in a 65-year-old male with a history of previous mediastinal tumor resection. CT imaging revealed a recurrent tumor extending into the left pleura. The patient underwent a posterolateral thoracotomy for complete tumor excision and limited replacement of the descending aorta. Postoperative recovery was smooth, and histology confirmed dedifferentiated LPS (G2) with areas of highly differentiated LPS. LPSs encompass a heterogeneous group of tumors with various subtypes, including atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLPS), dedifferentiated liposarcoma (DDLPS), myxoid liposarcoma (MLPS), and pleomorphic liposarcoma (PLPS). Treatment primarily involves complete surgical resection, while the roles of radiotherapy and chemotherapy remain debated. Immunotherapy shows potential benefits, particularly for DDLPS patients expressing PD-L1. Prognosis varies significantly by subtype, with DDLPS and PLPS associated with poorer outcomes compared to MLPS and ALT/WDLPS. Long-term follow-up is crucial for managing LPSs due to their high recurrence rate. This case highlights the effectiveness of surgical intervention in recurrent intrathoracic LPSs and underlines the need for continued research into adjuvant therapies to improve patient outcomes.
脂肪肉瘤是一种罕见的起源于脂肪细胞的恶性肿瘤,主要发生在四肢和腹膜后,累及胸部的情况极为罕见。本病例报告详细介绍了一名65岁男性复发性胸内脂肪肉瘤的手术治疗及结果,该患者既往有纵隔肿瘤切除术史。CT成像显示复发性肿瘤延伸至左胸膜。患者接受了后外侧开胸手术以完整切除肿瘤并对降主动脉进行有限置换。术后恢复顺利,组织学证实为去分化脂肪肉瘤(G2),伴有高分化脂肪肉瘤区域。脂肪肉瘤包括一组异质性肿瘤,有多种亚型,包括非典型脂肪瘤性肿瘤/高分化脂肪肉瘤(ALT/WDLPS)、去分化脂肪肉瘤(DDLPS)、黏液样脂肪肉瘤(MLPS)和多形性脂肪肉瘤(PLPS)。治疗主要包括完整的手术切除,而放疗和化疗的作用仍存在争议。免疫疗法显示出潜在益处,特别是对于表达PD-L1的DDLPS患者。预后因亚型而异,与MLPS和ALT/WDLPS相比,DDLPS和PLPS的预后较差。由于脂肪肉瘤的复发率高,长期随访对于其管理至关重要。本病例突出了手术干预在复发性胸内脂肪肉瘤中的有效性,并强调需要继续研究辅助治疗以改善患者预后。